OPN1LW Membrane Protein Introduction

Introduction of OPN1LW

OPN1LW is a gene existing on the X chromosome and encodes a long wave sensitive (LWS) opsin. It takes the responsibility to regulate perception of visible light in the yellow-green range on the visible spectrum (around 500-570nm). The gene contains 6 exons with variability that induces shifts in the spectral range. OPN1LW is subject to homologous recombination with OPN1MW, as the two have very similar genomes. These recombinations can lead to various vision problems, such as red-green colorblindness and blue monochromacy. The protein encoded by OPN1LW is a G-protein coupled receptor with embedded 11-cis-retinal, whose light excitation causes a cis-trans conformational change that begins the process of chemical signaling to the brain.

Basic Information of OPN1LW
Protein Name	Long-wave-sensitive opsin 1
Gene Name	OPN1LW
Aliases	Red cone photoreceptor pigment, Red-sensitive opsin (ROP)
Organism	Homo sapiens (Human)
UniProt ID	P04000
Transmembrane Times	7
Length (aa)	364
Sequence	MAQQWSLQRLAGRHPQDSYEDSTQSSIFTYTNSNSTRGPFEGPNYHIAPRWVYHLTSVWMIFVVTASVFTNGLVLAATMKFKKLRHPLNWILVNLAVADLAETVIASTISIVNQVSGYFVLGHPMCVLEGYTVSLCGITGLWSLAIISWERWLVVCKPFGNVRFDAKLAIVGIAFSWIWSAVWTAPPIFGWSRYWPHGLKTSCGPDVFSGSSYPGVQSYMIVLMVTCCIIPLAIIMLCYLQVWLAIRAVAKQQKESESTQKAEKEVTRMVVVMIFAYCVCWGPYTFFACFAAANPGYAFHPLMAALPAYFAKSATIYNPVIYVFMNRQFRNCILQLFGKKVDDGSELSSASKTEVSSVSSVSPA

Function of OPN1LW Membrane Protein

OPN1LW is a red-sensitive opsin. It resides in disks of the outer segment of LWS cone cells which mediate photopic vision along with MWS and SWS cones. Cone representation in the retina is not as big as rod representation, with the majority of cones localizing in the fovea. The 11-cis-retinal chromophore within the opsin protein becomes excited with reaching of LWS opsin spectral range. This excitation can bring about a conformational change in the protein and then trigger a series of chemical reactions. This reaction series transfers from the LWS cone cells into horizontal cells, bipolar cells, amacrine cells, and finally ganglion cells before continuing to the brain via the optic nerve. Ganglion cells compile the signal from the LWS cones with all other cone signals that occurred in response to the light that was seen and pass the overall signal into the optic nerve.

OPN1LW Membrane Protein Introduction Fig.1 Structure of OPN1LW.

Application of OPN1LW Membrane Protein in Literature

Li J., et al. Unique variants in OPN1LW cause both syndromic and nonsyndromic X-linked high myopia mapped to MYP1. Invest Ophthalmol Vis Sci. 2015, 56(6):4150-4155. PubMed ID: 26114493

The study suggests that special variants in OPN1LW can bring about both syndromic and nonsyndromic X-linked high myopia mapped to MYP1.
Buena-Atienza E., et al. De novo intrachromosomal gene conversion from OPN1MW to OPN1LW in the male germline results in blue cone monochromacy. Sci Rep. 2016, 6:28253. PubMed ID: 27339364

This article establishes intrachromosomal gene conversion in the male germline as an underlying mechanism and postulates gene conversion in the OPN1LW/OPN1MW genes.
Gardner J.C., et al. A novel missense mutation in both OPN1LW and OPN1MW cone opsin genes causes X-linked cone dystrophy (XLCOD5). Adv Exp Med Biol. 2012, 723:595-601. PubMed ID: 22183383

This article shows that mutations in the OPN1LW/OPN1MW cone opsin gene array can cause a spectrum of phenotypes, from color blindness to progressive cone dystrophy (XLCOD5).
Zahid S., et.al. OPN1LW and OPN1MW. Retinal Dystrophy Gene Atlas. 2018, 171-173.

This book shows mutations in OPN1LW and OPN1MW will cause a wide array of X-linked conditions ranging from red-green dyschromatopsia, blue cone monochromacy (BCM), cone/cone-rod dystrophy, and high myopia.
Cideciyan A.V., et al. Human cone visual pigment deletions spare sufficient photoreceptors to warrant gene therapy. Hum Gene Ther. 2013, 24(12):993-1006. PubMed ID: 24067079

The article suggests that human cones in patients with deletions in the red/green opsin gene array can survive in reduced numbers with limited outer segment material, suggesting the potential value of gene therapy for BCM.

OPN1LW Preparation Options

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